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Eculizumab Modifies Outcomes in Adults with Atypical Hemolytic Uremic Syndrome with Acute Kidney Injury.
- Published in:
- 2018
- By:
- Publication type:
- journal article
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation.
- Published in:
- 2010
- By:
- Publication type:
- journal article
Role of factor H-related protein 3 in Pseudomonas aeruginosa bloodstream infections.
- Published in:
- Frontiers in Immunology, 2024, p. 1, doi. 10.3389/fimmu.2024.1449003
- By:
- Publication type:
- Article
The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity.
- Published in:
- Frontiers in Immunology, 2022, v. 13, p. 1, doi. 10.3389/fimmu.2022.883743
- By:
- Publication type:
- Article
Eculizumab in dense-deposit disease after renal transplantation.
- Published in:
- 2014
- By:
- Publication type:
- Report
Eculizumab long-term therapy for pediatric renal transplant in aHUS with <i>CFH/CFHR1</i> hybrid gene.
- Published in:
- 2014
- By:
- Publication type:
- Report
Laforin, the most common protein mutated in Lafora disease, regulates autophagy.
- Published in:
- Human Molecular Genetics, 2010, v. 19, n. 14, p. 2867, doi. 10.1093/hmg/ddq190
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- Publication type:
- Article
The disease-protective complement factor H allotypic variant Ile62 shows increased binding affinity for C3b and enhanced cofactor activity.
- Published in:
- Human Molecular Genetics, 2009, v. 18, n. 18, p. 3452, doi. 10.1093/hmg/ddp289
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- Publication type:
- Article
Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.
- Published in:
- Human Molecular Genetics, 2005, v. 14, n. 8, p. 1107, doi. 10.1093/hmg/ddi099
- By:
- Publication type:
- Article
Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.
- Published in:
- Human Molecular Genetics, 2005, v. 14, n. 5, p. 703, doi. 10.1093/hmg/ddi066
- By:
- Publication type:
- Article
Laforin, the dual-phosphatase responsible for Lafora disease, interacts with R5 (PTG), a regulatory subunit of protein phosphatase-1 that enhances glycogen accumulation.
- Published in:
- Human Molecular Genetics, 2003, v. 12, n. 23, p. 3161, doi. 10.1093/hmg/ddg340
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- Publication type:
- Article
Complement Factor H Is Expressed in Adipose Tissue in Association With Insulin Resistance.
- Published in:
- Diabetes, 2010, v. 59, n. 1, p. 200, doi. 10.2337/db09-0700
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- Publication type:
- Article
Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome).
- Published in:
- International Journal of Molecular Sciences, 2021, v. 22, n. 12, p. 6608, doi. 10.3390/ijms22126608
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- Publication type:
- Article
Genetic and environmental factors influencing the human factor H plasma levels.
- Published in:
- Immunogenetics, 2004, v. 56, n. 2, p. 77, doi. 10.1007/s00251-004-0660-7
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- Publication type:
- Article
Genetic determinants of variation in the plasma levels of the C4b-binding protein (C4BP) in Spanish families.
- Published in:
- Immunogenetics, 2003, v. 54, n. 12, p. 862, doi. 10.1007/s00251-003-0537-1
- By:
- Publication type:
- Article
High Complement Factor H-Related (FHR)-3 Levels Are Associated With the Atypical Hemolytic-Uremic Syndrome-Risk Allele CFHR3*B.
- Published in:
- Frontiers in Immunology, 2018, p. 1, doi. 10.3389/fimmu.2018.00848
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- Publication type:
- Article
Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease.
- Published in:
- EMBO Molecular Medicine, 2017, v. 9, n. 7, p. 906, doi. 10.15252/emmm.201707608
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- Publication type:
- Article
Mutational spectrum of the EPM2A gene in progressive myoclonus epilepsy of Lafora: high degree of allelic heterogeneity and prevalence of deletions.
- Published in:
- European Journal of Human Genetics, 2000, v. 8, n. 12, p. 946, doi. 10.1038/sj.ejhg.5200571
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- Publication type:
- Article
Thrombotic microangiopathy in patients with malignant hypertension.
- Published in:
- Nephrology Dialysis Transplantation, 2023, v. 38, n. 5, p. 1217, doi. 10.1093/ndt/gfac248
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- Publication type:
- Article
Crystal Structure of Glyceraldehyde-3-Phosphate Dehydrogenase from the Gram-Positive Bacterial Pathogen A vaginae, an Immunoevasive Factor that Interacts with the Human C5a Anaphylatoxin.
- Published in:
- Frontiers in Microbiology, 2017, v. 8, p. 1, doi. 10.3389/fmicb.2017.00541
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- Publication type:
- Article
Testing the Activity of Complement Convertases in Serum/Plasma for Diagnosis of C4NeF-Mediated C3 Glomerulonephritis.
- Published in:
- Journal of Clinical Immunology, 2016, v. 36, n. 5, p. 517, doi. 10.1007/s10875-016-0290-5
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- Publication type:
- Article
Functional and structural characterization of four mouse monoclonal antibodies to complement C3 with potential therapeutic and diagnostic applications.
- Published in:
- European Journal of Immunology, 2017, v. 47, n. 3, p. 504, doi. 10.1002/eji.201646758
- By:
- Publication type:
- Article
Increased Endoplasmic Reticulum Stress and Decreased Proteasomal Function in Lafora Disease Models Lacking the Phosphatase Laforin.
- Published in:
- PLoS ONE, 2009, v. 4, n. 6, p. 1, doi. 10.1371/journal.pone.0005907
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- Publication type:
- Article
Riñón, hipertensión y activación del complemento. En búsqueda de nuevas dianas terapéuticas.
- Published in:
- Nefrologia, 2019, v. 39, n. 2, p. 111, doi. 10.1016/j.nefro.2018.10.002
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- Publication type:
- Article
Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso.
- Published in:
- Nefrologia, 2015, v. 35, n. 5, p. 421, doi. 10.1016/j.nefro.2015.07.005
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- Publication type:
- Article
Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso.
- Published in:
- Nefrologia, 2013, v. 33, n. 1, p. 27, doi. 10.3265/Nefrologia.pre2012.Nov.11781
- By:
- Publication type:
- Article
Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation.
- Published in:
- Kidney International, 2012, v. 82, n. 10, p. 1084, doi. 10.1038/ki.2012.250
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- Publication type:
- Article
Complement factor H variants I890 and L1007 while commonly associated with atypical hemolytic uremic syndrome are polymorphisms with no functional significance.
- Published in:
- 2012
- By:
- Publication type:
- journal article
Complement factor H variants I890 and L1007 while commonly associated with atypical hemolytic uremic syndrome are polymorphisms with no functional significance.
- Published in:
- Kidney International, 2012, v. 81, n. 1, p. 56, doi. 10.1038/ki.2011.291
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- Publication type:
- Article
The crystal structure of iC3b-CR3 αI reveals a modular recognition of the main opsonin iC3b by the CR3 integrin receptor.
- Published in:
- Nature Communications, 2022, v. 13, n. 1, p. 1, doi. 10.1038/s41467-022-29580-2
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- Publication type:
- Article
How novel structures inform understanding of complement function.
- Published in:
- Seminars in Immunopathology, 2018, v. 40, n. 1, p. 3, doi. 10.1007/s00281-017-0643-z
- By:
- Publication type:
- Article
C3 glomerulopathy--associated CFHR1 mutation alters FHR oligomerization and complement regulation.
- Published in:
- Journal of Clinical Investigation, 2013, v. 123, n. 7, p. 2434, doi. 10.1172/JCI68280
- By:
- Publication type:
- Article
C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation.
- Published in:
- 2013
- By:
- Publication type:
- journal article
C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation.
- Published in:
- Journal of Clinical Investigation, 2013, v. 123, n. 6, p. 2334, doi. 10.1172/JCI68280
- By:
- Publication type:
- Article
The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models.
- Published in:
- 2007
- By:
- Publication type:
- Other
m.6267G>A: a recurrent mutation in the human mitochondrial DNA that reduces cytochrome c oxidase activity and is associated with tumors.
- Published in:
- Human Mutation, 2006, v. 27, n. 6, p. 575, doi. 10.1002/humu.20338
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- Publication type:
- Article
The structure of Leptospira interrogans GAPDH sheds light into an immunoevasion factor that can target the anaphylatoxin C5a of innate immunity.
- Published in:
- Frontiers in Immunology, 2023, p. 1, doi. 10.3389/fimmu.2023.1190943
- By:
- Publication type:
- Article
Characteristics, management and outcomes of atypical haemolytic uraemic syndrome in kidney transplant patients: a retrospective national study.
- Published in:
- Clinical Kidney Journal, 2021, v. 14, n. 4, p. 1173, doi. 10.1093/ckj/sfaa096
- By:
- Publication type:
- Article
Anti-factor H antibody affecting factor H cofactor activity in a patient with dense deposit disease.
- Published in:
- Clinical Kidney Journal, 2012, v. 5, n. 2, p. 133, doi. 10.1093/ckj/sfs002
- By:
- Publication type:
- Article
Assessment of the interaction of human complement regulatory proteins with group A Streptococcus. Identification of a high-affinity group A Streptococcus binding site in FHL-1.
- Published in:
- European Journal of Immunology, 2000, v. 30, n. 4, p. 1243, doi. 10.1002/(SICI)1521-4141(200004)30:4<1243::AID-IMMU1243>3.0.CO;2-D
- By:
- Publication type:
- Article
The phosphatase activity of laforin is dispensable to rescue Epm2a−/− mice from Lafora disease.
- Published in:
- Brain: A Journal of Neurology, 2014, v. 137, n. 3, p. 806, doi. 10.1093/brain/awt353
- By:
- Publication type:
- Article
Genetic variability shapes the alternative pathway complement activity and predisposition to complement‐related diseases.
- Published in:
- Immunological Reviews, 2023, v. 313, n. 1, p. 71, doi. 10.1111/imr.13131
- By:
- Publication type:
- Article
The Relevance of the MCP Risk Polymorphism to the Outcome of aHUS Associated With C3 Mutations. A Case Report.
- Published in:
- Frontiers in Immunology, 2020, v. 11, p. 1, doi. 10.3389/fimmu.2020.01348
- By:
- Publication type:
- Article
Corrigendum: High Complement Factor H-Related (FHR)-3 Levels Are Associated With the Atypical Hemolytic-Uremic Syndrome-Risk Allele CFHR3 <sup>*</sup> B.
- Published in:
- 2020
- By:
- Publication type:
- Correction Notice
Blocking Complement Factor B Activation Reduces Renal Injury and Inflammation in a Rat Brain Death Model.
- Published in:
- Frontiers in Immunology, 2019, v. 10, p. 1, doi. 10.3389/fimmu.2019.02528
- By:
- Publication type:
- Article
Mechanism suppressing glycogen synthesis in neurons and its demise in progressive myoclonus epilepsy.
- Published in:
- Nature Neuroscience, 2007, v. 10, n. 11, p. 1407, doi. 10.1038/nn1998
- By:
- Publication type:
- Article
Crystal structure of human homogentisate dioxygenase.
- Published in:
- Nature Structural Biology, 2000, v. 7, n. 7, p. 542
- By:
- Publication type:
- Article
Case Report: Combined Liver-Kidney Transplantation to Correct a Mutation in Complement Factor B in an Atypical Hemolytic Uremic Syndrome Patient.
- Published in:
- Frontiers in Immunology, 2021, v. 12, p. 1, doi. 10.3389/fimmu.2021.751093
- By:
- Publication type:
- Article
Factor H-related protein 1 promotes complement-mediated opsonization of Pseudomonas aeruginosa.
- Published in:
- Frontiers in Cellular & Infection Microbiology, 2024, p. 01, doi. 10.3389/fcimb.2024.1328185
- By:
- Publication type:
- Article
A novel protein tyrosine phosphatase gene is mutated in progressive myoclonus epilepsy of the Lafora type (EPM2).
- Published in:
- Human Molecular Genetics, 1999, v. 8, n. 2, p. 345, doi. 10.1093/hmg/8.2.345
- By:
- Publication type:
- Article